hemangioma sam 3_2

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Objectives

Introduction Definition Classification Epidemiology Patho-physiology Clinical Presentation Investigations and Diagnosis Course and Prognosis Management

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Introduction

Hemangiomas are tumors identifiedby rapid endothelial cell proliferation

in inf ancy, followed by involution over time.

The malformations have a normalendothelial cell growth cycle that affects the veins, the capillaries, or the lymphatics, and they do not involute.

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Definition

Hemangiomas are lesions that are not present at birth, they manifest within thefirst month of life.

They grow rapidly during the first year (proliferating phase), undergo slowspont aneous regression during childhood(involution phase), and remain st ablethereafter or to near complete resolution.

Vascular malformations are more st ableand f ail to regress.

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Classification

The present binary biologic classificationdistinguishes between vascular tumors and vascular malformations.

Vascular malformations are sub-classified according to the st ructuralcomponents into capillary, venous,lymphatic, arterial, or combined forms.

Both vascular tumors and malformationscan be separated into slow-flow or f ast-flow types.

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`

 Vasoformative Tumor New Nomenclature Old Nomenclature

 Vascular tumors( i.e. Hemangiomas)

Capillary hemangioma St rawberry hemangioma

Juvenile hemangioma

Cavernous hemangioma

Mixed hemangioma Parotid hemangioma

 Vascular malformations

 Venous malformation Cavernous hemangioma

Hemangiomatosis

Int ramuscular venous malformation Int  ramuscular hemangioma

Capillary malformation Capillary hemangioma

Port-wine st ain

 Arteriovenous malformation Arteriovenous hemangioma Arterial angioma Arteriovenous aneurysm

Cirsoid angiomaRed angiomaSerpentine aneurysm

Lymphatic malformation Capillary lymphangiomaCavernous lymphangiomaLymphangiomaCystic hygroma

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Hemangiomas³vascular tumors´

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Hemangioma of infancy

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Definition

HEMANGIOMA OF INFANCY (HI) ;(Formerly st rawberry, cherry, capillaryhemangioma):

Benign vascular neoplasms that have a characteristic clinical course marked by earlyproliferation and followed by spont aneousinvolution.

Most common tumors of inf ancy, andmedically insignificant. Rarely, may beassociated with one or more underlyingcongenit al anomalies.

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Epidemiology

HEMANGIOMA OF INFANCY (HI) :

Is the most common tumor of infancy.The incidence in newborns is between 1 and 2.5%; most commonly in whiteinf ants. Of which 30% present at birth and70% at the first several weeks of life.Females are affected more often thanmales by a ratio of 3:1.

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Pathophysiology

HEMANGIOMA OF INFANCY (HI) :

Inf antile hemangiomas are composed of proliferating, plump endothelial cells.

Early in proliferation, the cells are indisarray. But, with time, they form vascular 

spaces and channels abundant with bloodcells.

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These benign-appearing endothelial cellsproduce limited basementmembrane structures.

Hemangiomas assume a lobulararchitecture as proliferation slows andends.

Mast cells appear to affect thisprocess.They also have been found inhigh concent rations during involution.

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Takahashi said:

That during the third t rimester of 

fet al development, immatureendothelial cells coexist withimmature pericytes, which maint aintheir proliferative capacity for a 

limited period during postnat al life.

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Histopathology of a proliferating infantile hemangioma with plump

endothelial cells in the dermis.

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Clinical feature

80% are focal and solit ary.

60% of cut aneous heamangiomas occur 

on the head and neck. Other sites: liver, GIT, larynx, CNS,

pancreas, gall bladder, thymus, spleen,lymph nodes, lung, urinary bladder, and

adrenal glands.

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Contd.

Early proliferating inf antilehemangiomas include: 

Blanching of the involved skin,followed by fine telangiect asias, and

then a red or crimson (purplish-red) macule or papule that often is

surrounded by a f aint halo of vascular blanching.

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Size: Most reach a maximum size of 0.5-5 cm, to greater than 20 cm in

diameter.

Most remain well circumscribed andfocal. A minority may be segment al innature.

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Investigations and Diagnosis

Dermatopathology shows variousamount of proliferation of endothelial

cells in dermis and subcut aneoustissue.

Increased GLUT-I immunoreactivity,but not in vascular malformation.

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Course and prognosis

CHI usually develops at birth or soonafter birth (first four weeks).

They may proliferates until 9 to 12months of age.

Most are spont aneously resolved:

 Age 5 years: 50% resolution

 Age 7 years: 70% resolution

 Age 9 years: 90% resolution

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Contd.

80% involutes without any residual skinchange at site of lesion.

Residual skin changes include at rophy,depigment ation, and scarring mayoccur.

Deeper lesions of mucous membranes

may not involute completely.

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Contd.

CHI may associated with:

Platelet ent rapment 

Thrombocytopenia DIC

Rarely, death secondary to severehemorrhage or heart f ailure.

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Management

The vast ma jority of inf antilehemangiomas do not require anymedical or surgical intervention becausespont aneous resolution gives lessscaring and the best cosmetic results.

Few cases may require medical or 

surgical t reatment.

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Medical treatment

Glucocorticosteroids (topical,int ralesional, and oral)

Interferon-alfa Beta-blockers (propranolol); have

recently been shown to induceinvolution of inf antile hemangiomas.

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Surgical treatment

Laser surgery is beneficial in t reatingboth proliferating and residual vesselsf rom hemangiomas.

The flash lamp-pumped pulsed-dyelaser has become the most widely usedlaser for selective ablation of vascular 

tissue in childhood.

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Surgical excision of involuted

hemangiomas may be used todecrease cut aneous defects resultingf rom them.

Surgical treatment

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Cavernoushemangioma

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Overview

Less common and lesscircumscribed than the capillaryvariety and more f requently involve

deep st ructures.

Rarely, giant forms occur that affect large subcut aneous areas of 

the f ace, ext remities, or other regionsof the body.

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Clinical feature

Large, irregular, deep dermal andsubcut aneous blood-filled channels that impart a purplish discoloration to theoverlying skin.

They are typically soft, poorly defined,and readily blanch with compression,

giving them a characteristic " bag ofworms" feel.

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The lesion may expand and darkenwith crying, when agit ated, or whenplaced in a dependent position .

They are readily compressible and fillslowly when released .

They lack a prominent pulsation; if 

they represent an arteriovenousmalformation, a thrill may be present .

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They are not present at birth but develop during childhood.

Usually asymptomatic and demonst ratethe same patterns of proliferation asthose of capillary lesions. However,involution is often incomplete.

Course and prognosis

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Cavernous hemangiomas maycomplicated with:

Ulceration ( the most common )

Bleeding, thrombocytopenia

High-out put heart f ailure

Secondary infection

Function problems (eg, airway, vision,hearing).

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There is no satisf actory t reatment except compression.

Some cases ( those withcompromised organ function ) mayrequire medical or surgicalintervention.

Management

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Steroids; high doses of systemic or int ralesional steroids are the first-line

t reatment, and a dramatic response isobserved in 30% of patients.

Interferon-alfa.

Medical treatment

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Complete surgical excision of theselesions offers the best chance of cure,

but followed by severe functionalimpairment to vit al functions.

Embolotherapy.

Cryosurgery.

Lasers; pulsed dye, argon and CO2.

Surgical treatment

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References

http:// emedicine.medsca p e.com / ar ticl e /108447 9-overvi ew

http:// emedicin e.medsc a p e.com/ ar ticl e /108384 9-ov er vi ew

FITZPATRICKS COLOR ATLAS AND SYN OP SIS OF  C LINI CAL DERM ATO LOG Y,SIXTH EDITION 

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